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1型遗传性运动感觉神经病(CMT1A)患儿足踝表现的演变

Evolution of foot and ankle manifestations in children with CMT1A.

作者信息

Burns Joshua, Ryan Monique M, Ouvrier Robert A

机构信息

Discipline of Paediatrics and Child Health, Faculty of Medicine, University of Sydney/Institute for Neuromuscular Research, Children's Hospital at Westmead, Locked Bag 4001, Westmead, Sydney, NSW 2145 Australia.

出版信息

Muscle Nerve. 2009 Feb;39(2):158-66. doi: 10.1002/mus.21140.

Abstract

We studied the timing and progression of foot and ankle changes in 81 children with genetically confirmed Charcot-Marie-Tooth disease type 1A (CMT1A) and determined their impact on motor function and walking ability. Foot deformity, weakness, pain, cramps, and instability were a common feature of CMT1A. Foot structure evolved toward pes cavus from early childhood to adolescence, although a subgroup with normal and planus feet remained. Foot strength increased with age, although compared to age-equivalent norms it declined from 4 years. Factors associated with evolving foot deformity included muscle weakness/imbalance, restricted ankle flexibility, and joint hypermobility. Regression modeling identified dorsiflexion weakness, global foot weakness, and difficulty toe-walking as independent predictors of motor dysfunction, while pes cavus and difficulty heel-walking were predictors of poor walking ability. Foot problems are present from the earliest stages of the disease and can have a negative impact on function. Early foot and ankle intervention may prevent long-term disability and morbidity in CMT1A.

摘要

我们研究了81例基因确诊为1A型遗传性运动感觉神经病(CMT1A)患儿足踝变化的时间和进展情况,并确定了这些变化对运动功能和行走能力的影响。足部畸形、无力、疼痛、痉挛和不稳是CMT1A的常见特征。从幼儿期到青春期,足部结构逐渐演变为高弓足,不过仍有一小部分足型正常和平足的患儿。足部力量随年龄增长而增强,尽管与同龄人标准相比,从4岁起就开始下降。与足部畸形演变相关的因素包括肌肉无力/失衡、踝关节活动度受限和关节过度活动。回归模型显示,背屈无力、全足无力和踮脚尖困难是运动功能障碍的独立预测因素,而高弓足和足跟行走困难则是行走能力差的预测因素。足部问题在疾病早期就已出现,并且可能对功能产生负面影响。早期的足踝干预可能预防CMT1A患儿出现长期残疾和发病情况。

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